[Management of chronic heart failure: clinical features and resource utilization among patients managed by the general practitioner. Pilot project in the province of Udine (Italy)]. / La gestione dello scompenso cardiaco cronico: caratteristiche cliniche ed assorbimento di risorse dei pazienti seguiti dal Medico di Medicina Generale. Studio pilota sul territorio della provincia di Udine (Italia).

RATIONALE: In the management of heart failure the general practitioner (GP) plays an important role. However, international studies proved that the GP differs in the management of these patients from the cardiologist. This pilot study aims at investigating if such differences persist in the Italian community. MATERIALS AND METHODS: Seventy patients with heart failure have been enrolled prospectively by 10 GPs in the Udine district (ASL 4). All of them have been evaluated at the first and subsequent visits, both with respect to clinical and instrumental parameters, overall resource consumption and quality of life. RESULTS: We observed a high degree of heterogeneity in the follow up patterns; a low coordination between GP and cardiologists in managing patients; several co-morbidities; high social burden; a good adherence to treatment guidelines; a moderate workload, subjectively evaluated from the GP. CONCLUSIONS: This experience motivated the need of further research in the field, and, from the point of view of the daily practice, the need of integrating hospital and community management of patients with heart failure.

Aortic pseudocoarctation. Description of two cases.

Aortic pseudocoarctation is a rare congenital anomaly characterized by elongation and kinking of the aortic arch. Regarded as a benign condition for a long time, several reports demonstrate that complications may occur so that a careful follow-up of patients affected by aortic pseudocoarctation is mandatory. In this context we describe two patients recently evaluated in our institution and review the literature to establish the most accurate diagnostic techniques (also considering newer noninvasive imaging modalities such as transesophageal echocardiography and nuclear magnetic resonance) and to evaluate the prognosis.

Idiopathic left ventricular aneurysm: an unusual substrate of ventricular fibrillation and ventricular tachycardia.

Idiopathic left ventricular aneurysm (LVA) is a very rare clinical condition. This article describes a patient with idiopathic LVA associated with episodes of ventricular tachycardia and ventricular fibrillation. Clinical and instrumental examinations did not reveal the pathogenesis of the aneurysm. The malignant clinical course suggests that an aggressive antiarrhythmic treatment, including ICD implantation, may be warranted.

Cardiac magnetic resonance imaging findings in patients with right ventricular outflow tract premature contractions.

AIMS: To assess prospectively the value of cardiac magnetic resonance imaging in patients with apparently idiopathic premature contractions arising from the right ventricular outflow tract. METHODS: We compared magnetic resonance imaging scans in 19 patients (13 males and six females, mean age 44 years) with frequent (> 100 per hour), monomorphic (left bundle branch block and inferior axis morphology) extrasystoles, and in 10 volunteers (four males and six females, mean age 36.7 years) without structural heart disease. Magnetic resonance imaging studies (1 or 1.5 Tesla) included spin-echo and gradient-echo sequences in the standard planes. The presence of structural and dynamic abnormalities of the right and left ventricles, such as reduced wall thickness, systolic bulging, and decreased systolic thickening, were evaluated. In addition, end-diastolic diameters of the right ventricular outflow tract were measured in the transverse plane. RESULTS: The dimensions of the right ventricular outflow tract were wider in patients with extrasystoles compared to the control group. Mean anteroposterior and transverse diameters were 39.6 +/- 4.6 mm vs 29.9 +/- 4.8 mm (P < 0.01) and 27.5 +/- 3.8 mm vs 20.5 +/- 2.5 mm (P < 0.01), respectively. Wall motion and morphological abnormalities were present in 16/19 (84%) patients, and were confined to the anterolateral wall in 15/16 cases. All normal subjects had normal magnetic resonance imaging findings (P = 0.008). CONCLUSIONS: Cardiac magnetic resonance imaging revealed that in patients with idiopathic right ventricular outflow tract premature contractions there was a higher rate of morphological and functional abnormalities of the right ventricular outflow tract than in the normal subjects. Large studies and long follow-up are needed to confirm whether these findings could help identify a localized form of arrhythmogenic cardiomyopathy, and its clinical significance.

Familial right ventricular dysplasia with biventricular involvement and inflammatory infiltration. Heart Muscle Disease Study Group.

The aetiology of right ventricular dysplasia/cardiomyopathy is presently unknown. A genetic background has been suggested, but myocarditis may play a part in its pathogenesis. Two familial cases of right ventricular dysplasia, one of whom had also a diagnosis of myocarditis, are reported. Both patients presented with ventricular arrhythmias. The father subsequently had a "flu-like" syndrome, heart failure, and biventricular dysfunction; "active" myocarditis was found at endomyocardial biopsy. Then the patient died suddenly. The daughter developed progressive biventricular dysfunction; then she was resuscitated from a cardiac arrest, and subsequently died suddenly. In both patients necropsy showed severe right ventricular atrophy and fibro-adipose substitution, associated with biventricular fibrosis. Inflammatory infiltration was also present in the first patient. This study shows the association of right ventricular dysplasia and myocarditis in the same family. These cases may represent a link between inherited and acquired ("inflammatory") forms of the disease.

Idiopathic left ventricular aneurysm: a clinical and pathological study of a new entity in the spectrum of cardiomyopathies.

In a prospective study on 340 cases with primary myocardial disease, eight patients (six males, two females, mean age 36 years, range 24-47) with an idiopathic left ventricular aneurysm were observed. All patients had normal coronary arteries, no angina or history of myocardial infarction; all but one had no risk factors for ischaemic heart disease; all had normal right ventricles; one patient had a history of familial dilated cardiomyopathy, two of 'flu-like' syndrome at the time of first symptom and two of alcohol abuse. All patients had ventricular tachycardia (VT), five sustained (of right bundle branch block morphology in three, and of different morphologies in two), three non-sustained. Patients with sustained VT had inducible VT (resembling the clinical one) on electrophysiological study. Electrocardiogram (ECG) showed an infarction pattern in three cases. Aneurysms were of limited size (2.1 +/- 1/11 segments on echocardiography) and were located in the septum, apex or posterior wall. Left ventricular ejection fraction (LVEF) was reduced (< 0.50) in six patients and was not correlated with the aneurysm size. The duration of illness was inversely correlated with LVEF (P < 0.05). Endomyocardial biopsy showed evidence of diffuse pathological changes in all cases (cell hypertrophy, myofibrillar lysis, mitochondriosis). During follow-up (64 +/- 32 months), patients were successfully treated with anti-arrhythmic drugs: no patients required surgical treatment to control ventricular arrhythmia. Considering the clinical and pathological features of idiopathic left ventricular aneurysm, this primary myocardial disease could be classified as a novel peculiar form of cardiomyopathy.

Right ventricular dysplasia: a clinical and pathological study of two families with left ventricular involvement.

BACKGROUND: Right ventricular dysplasia is a heart muscle disease of unknown cause that is often familial and is anatomically characterised by adipose or fibroadipose infiltration of the right ventricular myocardium. It is generally regarded as a selective disorder of the right ventricle. AIM: To investigate the prevalence and characteristics of left ventricular involvement in two families in which at least one member had right ventricular dysplasia confirmed at necropsy. METHODS AND RESULTS: Eight patients were found to be affected by right ventricular dysplasia. In three of them this was confirmed at necropsy. Echocardiography or angiography or both showed left ventricular involvement in seven. This ranged from localised wall motion abnormalities to moderate or severe left ventricular dysfunction. The disease was progressive in four cases. At necropsy the left ventricular myocardium showed predominant fibrosis and degenerative changes of the myocardial cells. There were areas of myocardial thinning with fatty infiltration at the apex in two patients. CONCLUSIONS: Familial right ventricular dysplasia can be a progressive disorder that affects the left ventricle. Advanced disease may be clinically confused with dilated cardiomyopathy.

[Beta blocking agents in the treatment of dilated cardiomyopathy: review of the literature and clinical experience with 67 patients]. / I betabloccanti nella terapia della cardiomiopatia dilatativa: revisione della letteratura ed esperienza clinica su 67 pazienti.

BACKGROUND: Several reports suggest that chronic beta blockade, most often with the beta 1 selective agent metoprolol, may improve hemodynamic and clinical function in patients with idiopathic dilated cardiomyopathy. However, controlled trials are limited and some studies have not shown beneficial effects in short term trials. Mechanisms of effectiveness are still debated and probably concern the capacity to avoid toxic myocardial damage by catecholamines, to induce receptor up-regulation, to contribute to the control of arrhythmias, to improve diastolic relaxation and other mechanisms. METHODS: After a revision of the literature, a preliminary clinical experience with metoprolol in dilated cardiomyopathy diagnosed according to the WHO definition is reported. Sixty-seven patients symptomatic for congestive heart failure or with complex ventricular arrhythmias associated with severe left ventricular dysfunction were submitted to test dose with metoprolol 5 mg bid for 2-7 days. All patients were completely studied, including coronary angiography and endomyocardial biopsy to exclude ischemic heart disease and active myocarditis. Four pts (6%) did not tolerate the first test dosage of metoprolol and twenty-two patients were excluded from analysis because of inadequate follow-up or because they were enrolled in an international trial. Forty-one patients underwent long-term treatment with metoprolol at a final mean dosage of 150 mg a day (range 50-200 mg) and are presently analyzed. The dosage was gradually increased during the first seven weeks. RESULTS: After 6 +/- 2 months and 12 +/- 2 months, 34 patients were stable or ameliorated (Group 1) and experienced an overall significant improvement of functional class (all pts in class I-II NYHA), of left and right ventricular ejection fraction (from 28 +/- 8.8% to 35.8 +/- 13.7% to 33.2 +/- 12.3% and from 38.6 +/- 11.8% to 42.4 +/- 5.8% to 45.2 +/- 12.2% respectively), of clinical signs of congestive heart failure, of cardiothoracic index, of left ventricular diameters and of arrhythmic pattern. Furthermore, the rate of ventricular couplets > 20/24h and of non-sustained ventricular tachycardia changed respectively from 46% and 54% to 4% and 21% at 12 +/- 2 months. None in Group 1 died nor is any waiting for heart transplantation. Eleven patients (Group 2) did not tolerate the drug acutely (4 pts) or deteriorated during the first 6 +/- 2 months (7 pts) of the treatment. In this group a worsening or an insignificant variation of all clinical and instrumental parameters was observed. During follow-up four patients of this group underwent heart transplantation (one died shortly after the operation because of infective complications), one died while waiting, two are currently waiting for heart transplantation, and three are still in heart failure (class III NYHA). No cases of sudden death occurred in any group of patients (15 pts with follow-up > 12 mo). CONCLUSIONS: Our uncontrolled study seems to confirm the beneficial effect of betablockers in a subgroup of patients with idiopathic dilated cardiomyopathy. The characterization of responders to this therapy is still undefined and will constitute the aim of future analyses.

Clinical aspects and haemodynamics in the follow-up of dilated cardiomyopathy and myocarditis.

Many studies on the natural history of dilated cardiomyopathy show high probability of death or of cardiac transplantation in a large percentage of patients. These studies have several methodological limitations. Our prospective study, carried out from 1971, and which evaluated 120 patients, showed improved survival in more recent years. Survival 3 years after diagnosis changed from 30% (1971-6/1981) to 88.4% (7/1986-1/1989). Thirty patients were investigated by haemodynamic exercise test to assess their haemodynamic behaviour during exercise, to evaluate the effects of pharmacological treatment and to define parameters of prognostic value. Different haemodynamic behaviours were observed. Haemodynamic investigation during exercise is useful to assess the effect of treatment and may have prognostic value. Myocarditis presents a spectrum of clinical symptoms and echocardiographic abnormalities. In patients with congestive heart failure left ventricular dysfunction is common. Patients with atrioventricular block or chest pain usually have good left ventricular function. During follow-up, improvement is possible but persistent left ventricular dysfunction is associated with a high mortality rate. When left ventricular function is good at presentation and does not deteriorate during follow-up the prognosis is good.

[The prognostic assessment of dilated cardiomyopathy: a follow-up of 138 patients]. / Valutazione prognostica della cardiomiopatia dilatativa: follow-up di 138 pazienti.

A total of 138 patients with idiopathic dilated cardiomyopathy were assessed and followed-up between July 1973 and October 1985 in order to evaluate prognostic risk indicators. Of these 102 were male and 36 were female with a mean age of 49.1 years. Each patient underwent physical examination, electrocardiography, echocardiography, cardiac catheterization and coronary angiography. The mortality rate was low for the first 3 years (respectively 8, 12.5 and 20%) while it was 57.5 at the end of the 5-year follow-up period. Univariate analysis at the time of the diagnosis revealed that five factors were predictive of the clinical course at the end of the fifth year: the NYHA functional class IV (p less than 0.0001); LV ejection fraction less than 0.30 (p less than 0.01); left ventricular failure, bi-ventricular failure and left ventricular end-diastolic pressure greater than 20 mmHg (p less than 0.05). Multivariate analysis was used to determine which combination of factors could most accurately predict survival. The most important prognostic factor was again the IV NYHA functional class (p less than 0.01) and, to a lesser degree, left ventricular end-diastolic pressure (p less than 0.05). The present study underlines that the survival rate of patients with dilated cardiomyopathy depends upon the selection of patients. This can explain the low 3-year mortality found in our patients. The NYHA functional class may be a useful practical guide for cardiac transplantation.

Clinical and pathologic study of familial dilated cardiomyopathy.

To evaluate the occurrence of familial cases of dilated cardiomyopathy (DC), 165 consecutive patients were studied. Diagnosis of myocardial disease was based on clinical, hemodynamic, bioptic, postmortem or a combination of these criteria. Twelve patients (7% of cases) showed evidence of myocardial disease in greater than or equal to 1 relative; 27 patients with myocardial disease were detected in the 12 families, but a suspected history of myocardial involvement was present in a further 16 cases. In 6 families proband and relatives were affected by DC (total 14 cases); in 1 of these families the disease began with an atrioventricular block. In 4 families the relatives showed the presence of myocarditis at the endomyocardial biopsy. In 2 families the relatives presented a right ventricular cardiomyopathy. The mode of inheritance was autosomal dominant in 7 families, recessive in 4; X-linked pattern may be hypothesized in 1. Nine patients died under the age of 45 years: 2 of sudden death, 6 of chronic heart failure and 1 of cerebral embolism. Familial transmission is not rare. Different modes of genetic transmission (autosomal dominant, recessive and X-linked) and different forms of myocardial disease suggest that familial DC may be a multifactorial disease.

[Histological substrate of ventricular tachycardias of the right bundle-branch block type (sensitive to verapamil)]. / Substrato istologico delle tachicardie ventricolari tipo blocco di branca destra (sensibili al verapamil).

In order to clarify the anatomic substrate of idiopathic ventricular tachycardia of right bundle-branch block morphology, responsive to verapamil, two patients with this type of arrhythmia were evaluated using hemodynamic studies, right ventricular endomyocardial biopsy as well as electrophysiological and pharmacological studies. Pressures, right and left ventriculography and coronary angiography were normal in both patients. Case 1 showed a prolonged sinus node recovery time and a histological pattern which was compatible with healed myocarditis. Case 2 presented a complete right bundle-branch block, intra-His conduction defects and histological features of aspecific cardiomyopathy. Our results underline the presence of diffuse ventricular histologic changes in patients with idiopathic ventricular tachycardia of right bundle-branch block morphology. These histologic changes seem unable to interfere with cardiac pump function but are sufficient to create significant abnormalities of impulse formation and conduction.

[Ambulatory ECG in cardiomyopathies]. / ECG dinamica nelle cardiomiopatie.

DILATED CARDIOMYOPATHY - Conduction and rhythm disturbances are frequent findings in dilated cardiomyopathy. 65 patients with dilated cardiomyopathy underwent 24-hour electrocardiographic monitoring: 95.4% showed ventricular arrhythmias, 80% complex ventricular arrhythmias and 44% runs of non-sustained ventricular tachycardia. Over 1000 ventricular extrasystoles in 24 hours were present in 44% of cases. Ventricular tachycardia and multiform and paired ventricular extrasystoles correlated significantly with the severity of cardiac dysfunction and with a worse prognosis. Patients undergoing antiarrhythmic therapy (amiodarone) showed a significant reduction in the number of ventricular extrasystoles and in the incidence of complex ventricular arrhythmias. HYPERTROPHIC CARDIOMYOPATHY - The high incidence of arrhythmias, particularly ventricular arrhythmias (approx. 70% of cases) in hypertrophic cardiomyopathy is well recognized: episodes of ventricular tachycardia are present in 20% of cases and are related to an elevated risk of sudden death. Antiarrhythmic treatment with amiodarone significantly reduces the number of ventricular extrasystoles and the episodes of ventricular tachycardia, may prevent sudden death and improve survival. RESTRICTIVE CARDIOMYOPATHY - Very little information is present in literature concerning this extremely rare form, in which every type of rhythm and conduction disturbance has been observed. CONCLUSIONS - Electrocardiographic monitoring is nowadays a very important tool in the management of cardiomyopathy patients, to identify possible patients at risk and to monitor the antiarrhythmic treatment.